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Mouse Anti-KCNH2 Recombinant Antibody (CBFYH-2603) (CBMAB-H3629-FY)

This product is mouse antibody that recognizes KCNH2. The antibody CBFYH-2603 can be used for immunoassay techniques such as: WB, IP, IF, ELISA.
See all KCNH2 antibodies

Summary

Host Animal
Mouse
Specificity
Mouse, Rat, Human
Clone
CBFYH-2603
Application
WB, IP, IF, ELISA

Basic Information

Specificity
Mouse, Rat, Human
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Potassium Voltage-Gated Channel Subfamily H Member 2
Introduction
This gene encodes a voltage-activated potassium channel belonging to the eag family. It shares sequence similarity with the Drosophila ether-a-go-go (eag) gene. Mutations in this gene can cause long QT syndrome type 2 (LQT2). Transcript variants encoding distinct isoforms have been identified.
Entrez Gene ID
Human3757
Mouse16511
Rat117018
UniProt ID
HumanQ12809
MouseO35219
RatO08962
Alternative Names
Potassium Voltage-Gated Channel Subfamily H Member 2; Potassium Voltage-Gated Channel, Subfamily H (Eag-Related), Member 2; Ether-A-Go-Go-Related Gene Potassium Channel 1; Voltage-Gated Potassium Channel Subunit Kv11.1; Ether-A-Go-Go-Related Protein 1; Eag-Related Protein 1; Eag Homolog; ERG-1; HERG1; H-ERG
Function
Pore-forming (alpha) subunit of voltage-gated inwardly rectifying potassium channel. Channel properties are modulated by cAMP and subunit assembly. Mediates the rapidly activating component of the delayed rectifying potassium current in heart (IKr) (PubMed:18559421, PubMed:26363003, PubMed:27916661).
Isoform A-USO
Has no channel activity by itself, but modulates channel characteristics by forming heterotetramers with other isoforms which are retained intracellularly and undergo ubiquitin-dependent degradation.
Isoform B-USO
Has no channel activity by itself, but modulates channel characteristics by forming heterotetramers with other isoforms which are retained intracellularly and undergo ubiquitin-dependent degradation.
Biological Process
Cardiac muscle contractionManual Assertion Based On ExperimentIMP:BHF-UCL
Cellular response to xenobiotic stimulusManual Assertion Based On ExperimentIDA:BHF-UCL
Membrane depolarization during action potentialManual Assertion Based On ExperimentIDA:BHF-UCL
Membrane repolarizationManual Assertion Based On ExperimentIMP:UniProtKB
Membrane repolarization during action potentialManual Assertion Based On ExperimentIDA:BHF-UCL
Membrane repolarization during cardiac muscle cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL
Membrane repolarization during ventricular cardiac muscle cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL
Negative regulation of potassium ion export across plasma membraneManual Assertion Based On ExperimentIDA:BHF-UCL
Negative regulation of potassium ion transmembrane transportManual Assertion Based On ExperimentIDA:BHF-UCL
Positive regulation of potassium ion transmembrane transportManual Assertion Based On ExperimentIDA:BHF-UCL
Positive regulation of transcription, DNA-templatedManual Assertion Based On ExperimentIMP:ARUK-UCL
Potassium ion export across plasma membraneManual Assertion Based On ExperimentIDA:BHF-UCL
Potassium ion homeostasisManual Assertion Based On ExperimentIDA:BHF-UCL
Potassium ion import across plasma membraneManual Assertion Based On ExperimentIDA:BHF-UCL
Potassium ion transmembrane transportManual Assertion Based On ExperimentIDA:BHF-UCL
Regulation of heart rate by cardiac conductionManual Assertion Based On ExperimentIMP:BHF-UCL
Regulation of heart rate by hormoneManual Assertion Based On ExperimentTAS:BHF-UCL
Regulation of membrane potentialManual Assertion Based On ExperimentIDA:BHF-UCL
Regulation of membrane repolarizationManual Assertion Based On ExperimentIDA:BHF-UCL
Regulation of potassium ion transmembrane transportManual Assertion Based On ExperimentIDA:BHF-UCL
Regulation of ventricular cardiac muscle cell membrane repolarizationManual Assertion Based On ExperimentIMP:BHF-UCL
Ventricular cardiac muscle cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL
Cellular Location
Cell membrane
Involvement in disease
Long QT syndrome 2 (LQT2):
A heart disorder characterized by a prolonged QT interval on the ECG and polymorphic ventricular arrhythmias. They cause syncope and sudden death in response to exercise or emotional stress, and can present with a sentinel event of sudden cardiac death in infancy. Deafness is often associated with long QT syndrome type 2.
Short QT syndrome 1 (SQT1):
A heart disorder characterized by idiopathic persistently and uniformly short QT interval on ECG in the absence of structural heart disease in affected individuals. It causes syncope and sudden death.
Topology
Cytoplasmic: 1-403
Helical: 404-424
Extracellular: 425-450
Helical: 451-471
Cytoplasmic: 472-495
Helical: 496-516
Extracellular: 517-520
Helical: 521-541
Cytoplasmic: 542-547
Helical: 548-568
Extracellular: 569-611
Pore-forming: 612-632
Extracellular: 633-638
Helical: 639-659
Cytoplasmic: 660-1159
PTM
Phosphorylated on serine and threonine residues. Phosphorylation by PKA inhibits ion conduction.

Aizawa, T., Wada, Y., Hasegawa, K., Huang, H., Imamura, T., Gao, J., ... & Horie, M. (2023). Non-missense variants of KCNH2 show better outcomes in type 2 long QT syndrome. Europace, 25(4), 1491-1499.

Zheng, Z., & Song, Y. (2023). Integrated analysis of the voltage-gated potassium channel-associated gene KCNH2 across cancers. BMC bioinformatics, 24(1), 51.

Lei, J., Wang, Q., Qu, T., Cha, L., Zhan, H., Xu, J., ... & Zhou, B. (2023). KCNH2 regulates the growth and metastasis of pancreatic cancer. Journal of Pancreatology, 6(3), 101-109.

Ng, C. A., Ullah, R., Farr, J., Hill, A. P., Kozek, K. A., Vanags, L. R., ... & Vandenberg, J. I. (2022). A massively parallel assay accurately discriminates between functionally normal and abnormal variants in a hotspot domain of KCNH2. The American Journal of Human Genetics, 109(7), 1208-1216.

Kekenes-Huskey, P. M., Burgess, D. E., Sun, B., Bartos, D. C., Rozmus, E. R., Anderson, C. L., ... & Delisle, B. P. (2022). Mutation-specific differences in Kv7. 1 (KCNQ1) and Kv11. 1 (KCNH2) channel dysfunction and long QT syndrome phenotypes. International Journal of Molecular Sciences, 23(13), 7389.

Ono, M., Burgess, D. E., Schroder, E. A., Elayi, C. S., Anderson, C. L., January, C. T., ... & Delisle, B. P. (2020). Long QT syndrome type 2: emerging strategies for correcting class 2 KCNH2 (hERG) mutations and identifying new patients. Biomolecules, 10(8), 1144.

Vanoye, C. G., & George, A. L. (2020). Decoding KCNH2 variants of unknown significance. Heart Rhythm, 17(3), 501-502.

De Zio, R., Gerbino, A., Forleo, C., Pepe, M., Milano, S., Favale, S., ... & Carmosino, M. (2019). Functional study of a KCNH2 mutant: novel insights on the pathogenesis of the LQT2 syndrome. Journal of Cellular and Molecular Medicine, 23(9), 6331-6342.

Engelbrechtsen, L., Mahendran, Y., Jonsson, A., Gjesing, A. P., Weeke, P. E., Jørgensen, M. E., ... & Hansen, T. (2018). Common variants in the hERG (KCNH2) voltage-gated potassium channel are associated with altered fasting and glucose-stimulated plasma incretin and glucagon responses. BMC genetics, 19, 1-9.

Bertalovitz, A. C., Badhey, M. L. O., & McDonald, T. V. (2018). Synonymous nucleotide modification of the KCNH2 gene affects both mRNA characteristics and translation of the encoded hERG ion channel. Journal of Biological Chemistry, 293(31), 12120-12136.

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For research use only. Not intended for any clinical use.

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